Maxillary undifferentiated carcinoma with rhabdoid features

The rhabdoid malignant tumor was individualized by Haas et al. as an independent entity within the kidney malignant neoplasms in 1981. From its histopathological characteristics, different cases of rhabdoid extra-renal tumor were documented. This aspect has been also recognized in a wide heterogeneous group of tumors (carcinoma, melanoma, mesothelioma and mesenchymal tumors). The diagnosis of extrarenal rhabdoid tumor is based on the presence of a proliferation of epithelioid cells with large nuclei, prominent nucleoli, abundant eosinophilic cytoplasm, and conspicuous cytoplasmic intermediate filaments, which compress the nuclei. In this article we present the case of a 50-year-old male patient with a malignant maxillary tumor with characteristic rhabdoid features. Its differential diagnosis will be discussed. The literature will also be reviewed.